CASE REPORT

Imaging characteristics and findings in thyroglossalduct cyst cancer and concurrent thyroid cancerLarry Shemen,1,2 Craig Harvey Sherman,3 Alyssa Yurovitsky4

1Department of Surgery,New York Presbyterian,Queens, New York, USA2Department of Head andNeck Surgery, Lenox HillHospital, New York, New York,USA3Manhattan DiagnosticRadiology, New York,New York, USA4Department of Pathology,Lenox Hill Hospital, New York,New York, USA

Correspondence toProfessor Larry Shemen,lshemen@gmail.com

Accepted 5 April 2016

To cite: Shemen L,Sherman CH, Yurovitsky A.BMJ Case Rep Publishedonline: [please include DayMonth Year] doi:10.1136/bcr-2016-215059

SUMMARYThyroglossal duct cyst cancer is rare, while synchronousthyroglossal duct cyst cancer with thyroid cancer is stillrarer. The radiographic features of this case areinstructive and crucial when evaluating a thyroglossalduct cyst.

BACKGROUNDSurgeons often are faced with patients having thyr-oglossal duct cysts. It is vital for the patient toundergo appropriate radiographic tests such assonogram and CT scan to evaluate if a cancer ispresent in the cyst or the thyroid.

CASE PRESENTATIONA 53-year-old man presented with a large midlineanterior neck mass of several months’ duration. Hedenied any radiation exposure or family history ofthyroid cancer. Examination disclosed a 5 cm neckmass situated below the hyoid, which moved withdeglutition.

INVESTIGATIONSTransverse sonographic view of the left thyroidlobe revealed a small 7 mm mainly echo-poornodule with a punctuate echogenic focus consistentwith microcalcification (figure 1). Colour Dopplerultrasound revealed mild vascularity at the periph-eral margin of this nodule (figure 2). Fine needleaspiration of the left thyroid mass showed papillarycancer.CT of the neck with contrast showed a

5.0×4.5×3.2 cm complex thyroglossal duct cystwith a 1.8 cm rest of thyroid tissue with coarseperipheral calcification (figures 3–5).

DIFFERENTIAL DIAGNOSISBenign thyroglossal duct cystMetastatic lymph nodeReactive lymph nodeDermoid

TREATMENTThe patient underwent a Sistrunk procedure andtotal thyroidectomy with paratracheal nodal dissec-tion (figure 6).Final pathology showed a 2.2 cm focus of papil-

lary thyroid cancer with a mixed papillary and fol-licular growth pattern in the thyroglossal duct cyst(figures 7–10), and a 5 mm focus of papillarycancer in the thyroid. Extrathyroidal extension waspresent. Five of eight lymph nodes were positivefor metastatic disease. The BRAF mutation was

found in both, the thyroid and thyroglossal ductcyst cancers. All of the margins were clear. Thepatient subsequently underwent radioimmuneiodine (RAI) treatment.

OUTCOME AND FOLLOW-UPThe patient was discharged the day after surgery.He has been well after his I131 treatment.

DISCUSSIONThyroglossal duct cysts usually present in youngadulthood. Thyroglossal duct cancer has beenreported by most authors as arising in 0.7–1.5% ofall thyroglossal duct cysts.1–3 Others have reportedhigher incidences of 4.9–6.5%.4 5

The mean age at diagnosis of thyroglossal ductcyst cancer is about 40 years for females and38 years for males (less than that for thyroidcancer).The suspicious clinical features of thyroglossal

duct cancer are a hard mass within the cyst wall,fixation to surrounding structures, sudden, rapidexpansion, presence of palpable lymph nodes oneither side, older age and a coincident firm thyroid

Figure 1 Thyroid cancer transverse sonogram:transverse sonographic view of the left thyroid lobereveals a small 7 mm mainly echo-poor nodule with apunctate echogenic focus, which may represent a smallcalcification.

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mass. The sonogram and/or CT should also include a carefulstudy of the thyroid and lateral neck. The radiographic featuresof a mural mass, microcalcification within the thyroglossal ductcyst and central necrosis within the cervical lymph nodes, aresuspicious for malignant transformation. Additionally, a concur-rent suspicious thyroid lesion may be detected.

The diagnosis can be confirmed with FNA. If there is acomplex pattern on the sonogram, the FNA should be takenfrom the solid portion of the thyroglossal duct cyst under sono-graphic guidance. However, several authors report relatively lowsensitivity of 56–62% for FNA of the thyroglossal duct cystresulting from the dilution by the cystic fluid.6 7 Certainly, a sus-picious thyroid lesion detected on sonogram should undergoFNA.

Figure 3 Thyroglossal duct cancer precontrast: transaxial precontrastCT view showing peripherally calcified mural nodule within thethyroglossal duct cyst.

Figure 4 Thyroglossal duct cancer coronal cut CT: coronal CT view ofthyroglossal duct cyst.

Figure 2 Thyroid cancer transverse sonogram colour Doppler: colourDoppler ultrasound reveals mild vascularity at the peripheral margin ofthe nodule.

Figure 5 Thyroglossal duct cancer sagittal cut CT: axial CT section(figure 3) and coronal (figure 4) and sagittal (figure 5) reformattedimages show a large, predominantly cystic midline anterior neck masstypical of a thyroglossal duct cyst. Peripherally calcified enhancingmural nodule is consistent with malignancy arising within a thyroidremnant contained in the cyst.

2 Shemen L, et al. BMJ Case Rep 2016. doi:10.1136/bcr-2016-215059

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The commonest histological type of malignant tumour is pap-illary cancer, but a follicular variant of papillary cancer is alsofound. Cervical nodal metastases can be found in 7–75% ofcases.3 4 8 This wide variation is likely explained by the diversityof opinion for elective neck dissection, and highlights the needfor careful clinical and sonographic examination of the lateralneck.

The universally accepted treatment for thyroglossal duct cystcancer is a Sistrunk procedure. The addition of total thyroidect-omy and nodal dissection is controversial. Where totalthyroidectomy was performed for thyroglossal duct cyst cancer,25–60% of the final pathology thyroid specimens showedthyroid cancer.9 10 Clearly, in this case with a positive preopera-tive FNA from the thyroid mass, a total thyroidectomy andnodal dissection were necessary. However, even if the thyroidcancer was not obvious preoperatively, in view of the high inci-dence of occult cancer within the thyroid, concurrent perform-ance of a total thyroidectomy would be advocated.

Recent studies have supported the independent origin of thyr-oglossal duct cyst carcinomas insofar as only 56% had a V600EBRAF mutation, and, of these, 80% had similar mutations inthe thyroid cancer. The lack of uniformity led the authors tobelieve that the origin of the neoplasm was from a thyroidremnant within the thyroglossal duct cyst as opposed to ametastasis.11

Figure 8 Thyroid cancer low power view: classic thyroid papillarycancer (H&E ×10).

Figure 9 Thyroid cancer high power view: classic nuclear features ofpapillary carcinoma including enlarged irregular nuclear with dispersedto optically clear-appearing chromatin, nuclear crowding andoverlapping, and nuclear grooves and inclusions (H&E ×40).

Figure 6 Operative specimen: operative specimen showingthyroglossal duct cyst and total thyroid.

Figure 7 Thyroid cancer low power view: classic thyroid papillarycancer (H&E ×10).

Figure 10 Follicular variant of thyroid papillary cancer with classicnuclear features (H&E ×40).

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Learning points

▸ Thyroglossal duct cancer may coexist with thyroid cancer.▸ Preoperative radiographic studies, including sonogram and

CT as well as FNA, are vital in the evaluation of suchpatients and can be helpful in suggesting the presence of acancer in the cyst or the thyroid.

▸ A Sistrunk procedure and total thyroidectomy arerecommended, to be then followed by RAI if necessary.

▸ In view of the high risk of lymph node metastases, cervicalnodal dissection±RAI treatment should be considered.

Acknowledgements The authors would like to thank Natalia Ryvkin, MLIS, AHIPHealth Education Library, New York–Presbyterian/Queens, for librarian assistance.

Contributors LS performed the surgery and wrote the text pertaining to theliterature search. CHS performed the CT scan and provided the detail regarding theradiological features. AY performed the pathology on the specimen and wrote thepathological description in the text.

Competing interests None declared.

Patient consent Obtained.

Provenance and peer review Not commissioned; externally peer reviewed.

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of 12 cases. Mayo Clin Proc 1997;72:315–19.3 Choi YM, Kim TY, Song DE, et al. Papillary thyroid cancer arising from a

thyroglossal duct cyst: a single institution experience. Endocr J 2013;60:665–70.

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6 Bardales RH, Suhrland MJ, Korourian S, et al. Cytologic findings in thyroglossal ductcarcinoma. Am J Clin Pathol 1996;106:615–19.

7 Shahin A, Burroughs FH, Kirby JP, et al. Thyroglossal duct cyst: a cytopathologicstudy of 26 cases. Diag Cytopathol 2005;33:365–9.

8 Hartl DM, Ghuzlan AA, Chami L, et al. High rate of multifocality and occult lymphnode metastases in papillary thyroid carcinoma arising in thyroglossal duct cysts.Ann Surg Oncol 2009;16:2595–601.

9 Pellegriti G, Lumera G, Malandrino P, et al. Thyroid cancer in thyroglossal duct cystsrequires a specific approach due to its unpredictable extension. J Clin EndocrinolMetab 2013;98:458–65.

10 Chrisoulidou A, Iliadou PK, Doumala E, et al. Thyroglossal duct cyst carcinomas: isthere a need for thyroidectomy. Hormones (Athens) 2013;12:522–8.

11 Rossi ED, Martini M, Straccia P, et al. Thyroglossal duct cyst cancer most likelyarises from a thyroid gland remnant. Virchows Arch 2014;465:67–72.

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